NR I328 IEXAM I2 ISTUDY IGUIDE
HEMATOLOGIC I DISORDERS: 7-10 I questions
• Identify I general I nursing I strategies I associated I with I altered I anemia
• Describe I the I morphology, I pathophysiology, I clinical I manifestations,
Itherapeutic I management I and I nursing I considerations I of I the
I followingIanemias:
o ***Laboratory Inorms: I6-12 Iyears Iof Iage
▪ Hemoglobin:
• 11.5-15.5 Ig/dl
▪ Hematocrit:
• 35-45%
▪ MCV I(mean Icorpuscular Ivolume)
• 77-95 IfL
• Measure Iof Iaverage Isize Iof IRBC
• ↓ I in I microcytic; I ↑ I in I macrocytic
▪ MCHC I (mean I corpuscular I Hgb I concentration)
• 31–37 Ig/dl
• Reflection Iof IHgb Iconcentration
• Refer Ito Icolor; Iin Ihypo-, Ivalues Iare I↓
▪ MCH I(mean Icorpuscular IHgb)
• 25–33 Ipg/cell
• Reflection Iof IHgb Iconcentration
• Refer Ito Icolor; Iin Ihypo-, Ivalues Iare I↓
o Anemia
▪ Condition Iwhere Ithe Ihemoglobin Icontent Iof Ithe Iblood Iis
Iinsufficient Ito Isatisfy Ibodily Ineeds
▪ Causes:
• Blood Iloss
• Accelerated I hemolysis
• Decreased Iproduction
▪ Typical IClinical IManifestations:
• Fatigue
• Dizziness
• Weakness
• Pallor
• ↑ Iheart Irate
• ↑ Ibreathing Irate
o Types:
▪ Iron-deficiency Ianemia—MOST ICOMMON IIN IPEDS
• Nutritional Ianemia
• Microcytic-hypochromic—small, I pale I RBC
• Who’s Iat Irisk?
o Varied
o Premature Ibabies Iat I↑ Irisk Ib/c Ithey Idon’t Iget Ithat
rd
Iblood Irush Ithe Imom Igives Ithe Ibaby Ilate Iin Ithat I3
Itrimester, Ib/c Ipremies Iare Iborn Iearly—this Iis Iwhy
Ipremature I infants I need I the I iron I supplements I at I 2-3
Imonths, I whereas Iterm I babies Idon’t I need I it I until I 4-6
Imonths
, o Multiples Iand Itwins Iat I ↑ I because Ionly Ifinite Iamount
Iof Iblood Iavailable Iin Ithat Ilast Irush Iof Iblood; Iso Inot
Iall Ithe Ibabies Iget Ienough Iiron
o Breast-fed I exclusively I babies I at I ↑ I risk—breast I milk
Idoesn’t Ihave Isame Iiron Icontent Ias Ifortified Iiron
Iformula
o Kiddos I with I chronic I blood I loss—GI I bleeders,
Ihemophiliacs
o Kiddos Iwith Ipoor Idietary Iintake; Iwhole Imilk Idoesn’t
Ihave Isame Iiron Iconcentration Ias Iiron Ifortified
Iformula
• Pathophysiology:
o Body I stores I iron Ithat I is I used I for I erythropoiesis
o Iron I necessary I mineral I for I erythropoiesis Ito I occur
o Not Ienough Iiron Ito Itransport Ito Ithe Ibone Imarrow Ito
Imake IRBCs
o Bone I marrow I still I makes I RBCs I but Ithey I are I very
Ismall I and I have I a I lower I hemoglobin
I concentration
• Clinical IManifestations:
o Typical Isigns Iof Ianemia Iplus:
▪ Irritability—due Ito Itissue Ihypoxia/O2 I craving
▪ Pica I(especially Iice, Idirt, Ichalk, Ipowder Ior
Ipure I starch)—eating I of I substances I that
I areInot Itypical Iof Ikiddos Ito Ieat; Ihowever
Ithis Icould Ibe Ihard Ito Idistinguish Ifrom Ithis
Ioccurring Inormally Iin Itoddlerhood
▪ Poor Imuscle Itone
▪ Growth Iretardation
▪ Headache
▪ Nail I bed I deformities I called?? I Spoon I nail
▪ Tachycardic; I possible I murmurs
• Lab IValues:
o Everything Iis I↓↓↓↓ Ib/c Iits Imicrocytic-hypochromic
• Implementations:
o EDUCATION***
▪ Prevention*
▪ High Iiron Ifoods*
• Foods Ithat Ia Itoddler Iwould Ieat; Ieggs,
Ibreads
• Whole Imilk Itakes Iup Iroom Iin Ibelly!!!
ISo I kiddo I may I not I intake I enough I iron-
Irich Ifood Ib/c Ithey’re Inot Ihungry;
Iremember I whole I milk I does I not I have
Isame Iiron Iconcentration Ilike Ifortified
Iformula Iso Iif I kiddo I is Idrinking Iequal
Iamounts I of I whole I milk I as I he/she I used
Ito Idrink Ithe Iformula Ihe/she Iis Inot
Igetting Ienough Iiron
▪ Iron I supplements
• Best Ion Ian Iempty Istomach
• Best Iwith IVitamin IC
• Don’t Igive Iwith Imilk
, • If Itaking Iefficiently Ithey Iwill Ihave
Iblack/green Itarry Istools
• Lab I values I best I indicator
• Use Ia Istraw Ito I prevent I staining I of
Iteeth
• Monitor Ifor Iconstipation I& Itreat
▪ Beta-Thallasemia
• Hemolytic Ianemia; Ibone Imarrow Iissue Iis Ithe Iunderlying
Ipatho—blood Icells Iare Iextremely Iunstable, IRBCs Ilyse Ivery
Ieasily
• Not I producing I correct I beta I chains I of I hgb—alpha I and
Igamma I attempt I to I take I over I and I produce I RBCs I that
I areIfragile Iand Iprone Ito Ilysing
• ***kiddos IBM Iis Inot Imaking Iright Ihemoglobin
Ichains—they I break I down I very I quickly I and I easily
• Microcytic-hypochromic
• Defective I synthesis I of I the I protein I component I of
Ihemoglobin
• Who’s I at I Risk
o Mediterranean Idescent—Italians Iand IGreeks
• Pathophysiology:
o Hemoglobin Isynthesis Iis Iimpaired
o Fragile IRBC Iw/short Ilife
o Severe Ianemia
• Types:
o Major
▪ Most Isevere
▪ Occurs I in I infancy
▪ If Ikiddo I doesn’t I receive Ifreq. Itransfusions
Iand I treatments I they I rarely I survive
I childhood
▪ Without Itreatment Ithey Idie Ibefore Iage I7
o Intermediate
▪ Develop I normally I into I adulthood
▪ Need Ito Ibe Itreated
▪ Delayed Ipuberty
o Minor
▪ Carry I the I trait, I don’t I have I it
▪ Have I normal I life-span
• Clinical IManifestations:
* occurring Ib/c Iof IBM Iinvolvement
o Frequent I epistaxis*
o Osteoporosis*
o Pathologic Ifractures*
o Chronic I CHF
o Myocardia I fibrosis
o Murmurs
, Hepatosplenomegaly—b/c I spleen I has I to
o
I workIharder Ito Iclean I up Idysfunctional I RBCs
o Diabetes I mellitus
o Darkening Iof Ithe Iskin—indication Iof Iiron Itoxicity
Ithat’s Ioccurring
• Therapeutic I Management:
o Frequent Iblood Itransfusion
▪ Subsequent I iron I chelation I therapy
▪ ***Enough Itransfusions Ito Imaintain Ithe IHgb
Igreater Ithan I9.5
▪ Monitor Ifor Iiron Ioverload
o Splenectomy
• Nursing IManagement:
o Promote Icompliance
o Assist Iw/ I coping Istrategies
o Observe I for I complications I of I multiple I transfusions
o Differentiate Ibetween IIron IDeficiency IAnemia I& IThalassemia
IDA Thalassemia
Ferritin ILevels
Serum Iiron
TIBC
MCHC
Hemoglobin
Electrophoresis
Ethnic IBackground
▪ Sickle-cell Ianemia
• Hemolytic I anemia
• Autosomal I recessive I condition I where I normal
I hemoglobinIis Ipartially Ior Icompletely Ireplaced Iby Ithe
Isickle-shaped, Iabnormal IS IHgb
• Won’t Isee Imanifestations Iuntil Iabout I6-12 Imonths Iafter
Ibirth Ib/c Ikiddo Istill Ihad Ifetal Ihemoglobin Icirculating; Ifetal
Ihemoglobin Idoes Inot Isickle!
• Who’s Iat IRisk?
• Pathophysiology:
o Biggest Itrigger Iis Ihypoxia—stress, Ihigh Ialtitudes,
Icold, Inot Ibreathing Iwell, Iexcessive Iexercises, Ilow
Ibody Ior Ienvironment Itemp, Ianesthesia—watch
Iclosely Iin IOR, Idehydration, Iinfection, Iacidosis
o Hemoglobin IS Ibecomes Ielongated Iand Irigid
o Cells Iclump Itogether Iand Iobstruct I capillary Iblood
Iflow
o Ischemia I and I tissue I infarction I occur I beyond I the
Iobstruction
o Hypoxia I continues I which I leads I to I tissue I infarcts—
Iischemia Iof Itissue Iis Iextremely Ipainful
• Crisis I experienced:
o Vaso-occlusive I crisis
▪ Norm/MC—typically Inon-life Ithreatening;
Iassociated Iwith Ia Ilot Iof Ipain
HEMATOLOGIC I DISORDERS: 7-10 I questions
• Identify I general I nursing I strategies I associated I with I altered I anemia
• Describe I the I morphology, I pathophysiology, I clinical I manifestations,
Itherapeutic I management I and I nursing I considerations I of I the
I followingIanemias:
o ***Laboratory Inorms: I6-12 Iyears Iof Iage
▪ Hemoglobin:
• 11.5-15.5 Ig/dl
▪ Hematocrit:
• 35-45%
▪ MCV I(mean Icorpuscular Ivolume)
• 77-95 IfL
• Measure Iof Iaverage Isize Iof IRBC
• ↓ I in I microcytic; I ↑ I in I macrocytic
▪ MCHC I (mean I corpuscular I Hgb I concentration)
• 31–37 Ig/dl
• Reflection Iof IHgb Iconcentration
• Refer Ito Icolor; Iin Ihypo-, Ivalues Iare I↓
▪ MCH I(mean Icorpuscular IHgb)
• 25–33 Ipg/cell
• Reflection Iof IHgb Iconcentration
• Refer Ito Icolor; Iin Ihypo-, Ivalues Iare I↓
o Anemia
▪ Condition Iwhere Ithe Ihemoglobin Icontent Iof Ithe Iblood Iis
Iinsufficient Ito Isatisfy Ibodily Ineeds
▪ Causes:
• Blood Iloss
• Accelerated I hemolysis
• Decreased Iproduction
▪ Typical IClinical IManifestations:
• Fatigue
• Dizziness
• Weakness
• Pallor
• ↑ Iheart Irate
• ↑ Ibreathing Irate
o Types:
▪ Iron-deficiency Ianemia—MOST ICOMMON IIN IPEDS
• Nutritional Ianemia
• Microcytic-hypochromic—small, I pale I RBC
• Who’s Iat Irisk?
o Varied
o Premature Ibabies Iat I↑ Irisk Ib/c Ithey Idon’t Iget Ithat
rd
Iblood Irush Ithe Imom Igives Ithe Ibaby Ilate Iin Ithat I3
Itrimester, Ib/c Ipremies Iare Iborn Iearly—this Iis Iwhy
Ipremature I infants I need I the I iron I supplements I at I 2-3
Imonths, I whereas Iterm I babies Idon’t I need I it I until I 4-6
Imonths
, o Multiples Iand Itwins Iat I ↑ I because Ionly Ifinite Iamount
Iof Iblood Iavailable Iin Ithat Ilast Irush Iof Iblood; Iso Inot
Iall Ithe Ibabies Iget Ienough Iiron
o Breast-fed I exclusively I babies I at I ↑ I risk—breast I milk
Idoesn’t Ihave Isame Iiron Icontent Ias Ifortified Iiron
Iformula
o Kiddos I with I chronic I blood I loss—GI I bleeders,
Ihemophiliacs
o Kiddos Iwith Ipoor Idietary Iintake; Iwhole Imilk Idoesn’t
Ihave Isame Iiron Iconcentration Ias Iiron Ifortified
Iformula
• Pathophysiology:
o Body I stores I iron Ithat I is I used I for I erythropoiesis
o Iron I necessary I mineral I for I erythropoiesis Ito I occur
o Not Ienough Iiron Ito Itransport Ito Ithe Ibone Imarrow Ito
Imake IRBCs
o Bone I marrow I still I makes I RBCs I but Ithey I are I very
Ismall I and I have I a I lower I hemoglobin
I concentration
• Clinical IManifestations:
o Typical Isigns Iof Ianemia Iplus:
▪ Irritability—due Ito Itissue Ihypoxia/O2 I craving
▪ Pica I(especially Iice, Idirt, Ichalk, Ipowder Ior
Ipure I starch)—eating I of I substances I that
I areInot Itypical Iof Ikiddos Ito Ieat; Ihowever
Ithis Icould Ibe Ihard Ito Idistinguish Ifrom Ithis
Ioccurring Inormally Iin Itoddlerhood
▪ Poor Imuscle Itone
▪ Growth Iretardation
▪ Headache
▪ Nail I bed I deformities I called?? I Spoon I nail
▪ Tachycardic; I possible I murmurs
• Lab IValues:
o Everything Iis I↓↓↓↓ Ib/c Iits Imicrocytic-hypochromic
• Implementations:
o EDUCATION***
▪ Prevention*
▪ High Iiron Ifoods*
• Foods Ithat Ia Itoddler Iwould Ieat; Ieggs,
Ibreads
• Whole Imilk Itakes Iup Iroom Iin Ibelly!!!
ISo I kiddo I may I not I intake I enough I iron-
Irich Ifood Ib/c Ithey’re Inot Ihungry;
Iremember I whole I milk I does I not I have
Isame Iiron Iconcentration Ilike Ifortified
Iformula Iso Iif I kiddo I is Idrinking Iequal
Iamounts I of I whole I milk I as I he/she I used
Ito Idrink Ithe Iformula Ihe/she Iis Inot
Igetting Ienough Iiron
▪ Iron I supplements
• Best Ion Ian Iempty Istomach
• Best Iwith IVitamin IC
• Don’t Igive Iwith Imilk
, • If Itaking Iefficiently Ithey Iwill Ihave
Iblack/green Itarry Istools
• Lab I values I best I indicator
• Use Ia Istraw Ito I prevent I staining I of
Iteeth
• Monitor Ifor Iconstipation I& Itreat
▪ Beta-Thallasemia
• Hemolytic Ianemia; Ibone Imarrow Iissue Iis Ithe Iunderlying
Ipatho—blood Icells Iare Iextremely Iunstable, IRBCs Ilyse Ivery
Ieasily
• Not I producing I correct I beta I chains I of I hgb—alpha I and
Igamma I attempt I to I take I over I and I produce I RBCs I that
I areIfragile Iand Iprone Ito Ilysing
• ***kiddos IBM Iis Inot Imaking Iright Ihemoglobin
Ichains—they I break I down I very I quickly I and I easily
• Microcytic-hypochromic
• Defective I synthesis I of I the I protein I component I of
Ihemoglobin
• Who’s I at I Risk
o Mediterranean Idescent—Italians Iand IGreeks
• Pathophysiology:
o Hemoglobin Isynthesis Iis Iimpaired
o Fragile IRBC Iw/short Ilife
o Severe Ianemia
• Types:
o Major
▪ Most Isevere
▪ Occurs I in I infancy
▪ If Ikiddo I doesn’t I receive Ifreq. Itransfusions
Iand I treatments I they I rarely I survive
I childhood
▪ Without Itreatment Ithey Idie Ibefore Iage I7
o Intermediate
▪ Develop I normally I into I adulthood
▪ Need Ito Ibe Itreated
▪ Delayed Ipuberty
o Minor
▪ Carry I the I trait, I don’t I have I it
▪ Have I normal I life-span
• Clinical IManifestations:
* occurring Ib/c Iof IBM Iinvolvement
o Frequent I epistaxis*
o Osteoporosis*
o Pathologic Ifractures*
o Chronic I CHF
o Myocardia I fibrosis
o Murmurs
, Hepatosplenomegaly—b/c I spleen I has I to
o
I workIharder Ito Iclean I up Idysfunctional I RBCs
o Diabetes I mellitus
o Darkening Iof Ithe Iskin—indication Iof Iiron Itoxicity
Ithat’s Ioccurring
• Therapeutic I Management:
o Frequent Iblood Itransfusion
▪ Subsequent I iron I chelation I therapy
▪ ***Enough Itransfusions Ito Imaintain Ithe IHgb
Igreater Ithan I9.5
▪ Monitor Ifor Iiron Ioverload
o Splenectomy
• Nursing IManagement:
o Promote Icompliance
o Assist Iw/ I coping Istrategies
o Observe I for I complications I of I multiple I transfusions
o Differentiate Ibetween IIron IDeficiency IAnemia I& IThalassemia
IDA Thalassemia
Ferritin ILevels
Serum Iiron
TIBC
MCHC
Hemoglobin
Electrophoresis
Ethnic IBackground
▪ Sickle-cell Ianemia
• Hemolytic I anemia
• Autosomal I recessive I condition I where I normal
I hemoglobinIis Ipartially Ior Icompletely Ireplaced Iby Ithe
Isickle-shaped, Iabnormal IS IHgb
• Won’t Isee Imanifestations Iuntil Iabout I6-12 Imonths Iafter
Ibirth Ib/c Ikiddo Istill Ihad Ifetal Ihemoglobin Icirculating; Ifetal
Ihemoglobin Idoes Inot Isickle!
• Who’s Iat IRisk?
• Pathophysiology:
o Biggest Itrigger Iis Ihypoxia—stress, Ihigh Ialtitudes,
Icold, Inot Ibreathing Iwell, Iexcessive Iexercises, Ilow
Ibody Ior Ienvironment Itemp, Ianesthesia—watch
Iclosely Iin IOR, Idehydration, Iinfection, Iacidosis
o Hemoglobin IS Ibecomes Ielongated Iand Irigid
o Cells Iclump Itogether Iand Iobstruct I capillary Iblood
Iflow
o Ischemia I and I tissue I infarction I occur I beyond I the
Iobstruction
o Hypoxia I continues I which I leads I to I tissue I infarcts—
Iischemia Iof Itissue Iis Iextremely Ipainful
• Crisis I experienced:
o Vaso-occlusive I crisis
▪ Norm/MC—typically Inon-life Ithreatening;
Iassociated Iwith Ia Ilot Iof Ipain
